First noted by Wernher in 1843, cystic lymphangioma is one of three kinds of lymphangioma. (Lymphangioma circumscriptum and cavernous lymphangioma are the other two types). When cystic lymphangioma is diagnosed in infants, it poses little immediate health threat, but is often treated for cosmetic reasons. In cases where it emerges early in the process of fetal development, though, cystic lymphangioma can result in fetal death due to excess fluid accumulation. Around half of all cases are identified at birth, and up to ninety percent of cases are symptomatic by the time a child reaches age two. While the vast majority of cases occur in fetuses and infants, cystic lymphangioma can affect people of all ages.
Cystic Lymphangioma Signs and Symptoms
Cystic lymphangioma may also be referred to as cystic hygroma, familial nuchal bleb, fetal cystic hygroma (FCH), or hygroma colli. It involves a malformation of the lymph nodes, and can affect any part of the body, although three-quarters of cystic lymphangioma tumors are found on the head or neck. Cystic lymphangioma results in the appearance of a soft, fluid-filled, yellowish, non-malignant tumor, which usually appears as swollen bubble underneath the skin. The tumor itself is referred to as a hamartoma.
Cystic Lymphangioma Causes and Risk Factors
The direct cause of cystic lymphangioma is a blockage of the lymphatic system as a fetus develops, although symptoms may not become visible until after the baby is born. This blockage, in turn, is thought to be caused by a number of factors, including maternal alcohol use and viral infections during pregnancy. Cases of cystic lymphangioma that emerge during the first two trimesters of pregnancy may be associated with genetic disorders like Turnerís syndrome, Noonan syndrome, and chromosome abnormalities such as trisomies 13, 18, and 21.
Cystic Lymphangioma Prognosis
The prognosis for fetuses that are diagnosed with cystic lymphangioma late in the pregnancy is generally good. However, it is more dangerous when fetuses develop the condition early, especially when a chromosome abnormality is present. When these early cases of cystic lymphangioma resolve themselves quickly (by 18-20 weeks of gestation), the prognosis is still moderate to good, but cases that are not resolved at this stage have a poor prognosis.
Cystic Lymphangioma Diagnosis
Prenatal cystic lymphangioma can be diagnosed using abdominal ultrasonography at around 10 weeks, or by inspection for postnatal cases. In cases where fetal cystic lymphangioma is present, and no other explanations present themselves, family medical history is checked, and amniocentesis or CVS procedures may be conducted to check for genetic disorders.
Cystic Lymphangioma Treatment
When cystic lymphangioma is diagnosed before birth, the doctor may recommend delivery by cesarean section if the tumor is large, and syringes may be used to drain the tumor after birth and prevent airway obstruction.
For cases of cystic lymphangioma that are not noticed until after delivery, the prognosis is excellent. These tumors present minimal health risks, and are typically treated only for cosmetic purposes. Since draining the tumor is not a permanent solution, surgical resection is often recommended in cases where no infection is present.